1. Common Pediatric malignancies
Leukemia
CNS tumors
Lymphomas
Wilms
Neuroblastoma
Rhabdomyosarcoma
Others

2. Neuroblastoma: Site of origin
Adrenals (abdomen) 50%
Paraspinal (abdomen) 24%
Mediastinum 20%
Cervical 4%
Pelvic 2%

3. Neuroblastoma
Malignancy in neural crest cells in sympathetic ganglia, adrenal medulla, chest, abdomen;
small round blue tumor cells
Nonmalignant form is ganglioneuroma
Clinical effects is related to tumor size and location
Genetic links/factors involved: N-myc oncogene, chromosome deletion

4. NB Incidence (7-10%) of childhood cancer 500 new US per year
Most common cancer in infants – accounts for 50% of cancer in NBs.
M:F ratio: 1.2:1
Average age is 18 months; 80% < 5;
May be a “Silent” tumor
The most common spontaneously regressing tumor
Most children present with advanced disease

5. Clinical Presentation
Hepatomegaly,
subcutaneous nodules,
orbital swelling,
Horner's syndrome
paraplegia,
anemia and thrombocytopenia
Paraneoplastic syndromes, VIP, (WADHA),

6. Clinical Presentation
Pain, abd mass, other masses, skin nodules
mediastinum; +/- spinal cord compression**
Metastatic to lymph nodes, bone, BM, liver
Fever and malaise; catecholamine secretion: HTN, sweats, irritability; diarrhea; opsoclonus-myoclonus; cerebellar ataxia

7. Diagnostic Workup CT/MRI to locate tumor; bone scan; MIBG; PET?
Labs (urinary catecholamines); VMA, HVA
Bilateral BMA and biopsy; chromosome studies
Serum ferritin, NSE, LDH

9. Neuroblastoma Staging
1 Localized tumor; complete excision
2A Unilateral, incomplete gross resection; negative microscopic nodes
2B Unilateral, positive ipsilateral nodes; negative contralateral
3 Across midline, or contralateral nodes
4 Dissemination: BM, liver, skin, bones
4S <1y: local stage with metastasis to BM, liver, skin

10. Treatment and Prognosis
Surgery: debulking or total removal; curative in low-stage disease; 2nd-look after other treatment
Chemotherapy – often platinum based multi-agent ~ stage
Radiotherapy: to primary tumor site; NB cells very radiosensitive; before or after surgery; emergency relief for cord compression, respiratory compromise, proptosis

11. Treatment cont’d Bone MarrowTransplant:
children with poor prognosis initially may be treated with high dose chemotherapy with autologous stem cell rescue(s);
BMT may be used with relapse
Prognosis: <1 best (75+% survival);
worst for children >2 with stage IV disease (10-20%);

12. Wilms tumor Primary tumors arising from the kidney, usually Wilms
Others: clear cell sarcoma, renal cell CA, lymphoma, PNET, rhabdoid, …
Wilms tumor pathology may be favorable or unfavorable depending on degree of anaplasia present;
prognosis and treatment is related to pathology

13. Incidence and Etiology
Renal tumors represent 7-10% of pediatrics cancer;
500 new US cases/yr
Peak age at 2-3; rare >5;
M:F 0.9:1.0 (unilateral)
1.5% familial in origin; associated with aniridia, hemihypertrophy, Genito-Urinary malformations
Genetic factors, deletion or translocations

14. BeckwithWiedemann syndrome
Omphalocele
Macroglossia
Gigantism
Exophthalmos
Hypoglycemia

15. Hemi-hypertrophy

16. Clinical Presentation
Asymptomatic abdominal mass found by family or on routine Physical Examination
Pain, malaise, hematuria in 20-30%; 25% with HTN; rare subcapsular hemorrhage, with rapid increase in size, anemia,
Metastasis to lungs, liver, regional nodes
7% bilateral, at diagnosis or later

17. Diagnostic Workup History and Physical examination
Labs, renal and hepatic function
Imaging studies: US to determine size and shape, vessel involvement, thrombi in major vessels; chest film/CT to check for metastasis
Liver, brain, and bone metastasis not routinely assessed unless indicated by Signs and Symptoms

19. Prognosis
Histology is most important prognostic factor (favorable histology vs. anaplastic)
Stage at diagnosis also crucial
Genetic factors
Age

20. Staging of Wilms Tumors
I Limited to kidney; complete resection
II Extent beyond kidney, but complete resected
III Residual tumor, confined to abdomen
IV Hematogenous metastasis (lung, liver, bone, brain) or lymph nodes outside abdomen
V Bilateral renal involvement at diagnosis
Tumor spill at time of surgery – considered stage III

21. Treatment and Prognosis
Surgery initially, with exam of contralateral kidney;
Pre-operative chemotherapy if intravascular spread or very large invasive tumors; if bilateral;

22. Treatment and Prognosis cont’d
Bilateral: pre-operative Chemotherapy; nephrectomy of worse side, partial on other
Chemotherapy:
RadioTherapy: port extended across midline to prevent scoliosis; if favorable histology, RT only for Stage III and IV;
Prognosis: <50% - 100% (stage/histology)